Kornitzer Jeffrey, Taha Firas, Segal Eric
1 Rutgers New Jersey Medical School, Newark, NJ, USA.
2 St. Joseph's Children's Hospital, Paterson, NJ, USA.
J Child Neurol. 2019 Aug;34(9):499-505. doi: 10.1177/0883073819840448. Epub 2019 Apr 23.
We present the diagnostic and clinical course of the first multicenter case series of pediatric patients with autoimmune encephalitis associated with N-type voltage-gated calcium channel antibodies.
Data from 2 university hospitals were retrospectively reviewed and records of 3 patients with autoimmune encephalitis associated with N-type voltage-gated calcium channel antibodies were evaluated.
The 3 pediatric patients (all female) had symptoms that spanned the clinical spectrum. All 3, however, had regression of expressive language and agitation. Neuroimaging in all 3 patients was normal; electroencephalographic (EEG) findings varied among the 3 patients. Positive titers against the N-type voltage-gated calcium channel antibody were found in their cerebrospinal fluid. Following administration of intravenous immunoglobulin, all 3 had improvement in their core presenting symptoms.
Autoimmune encephalitis associated with N-type voltage-gated calcium channel antibodies in the pediatric population presents with a wide clinical spectrum, although expressive language delay and agitation seem to be common symptoms. Treatment with intravenous immunoglobulin improves core symptoms.
我们展示了首例多中心儿童自身免疫性脑炎伴N型电压门控钙通道抗体病例系列的诊断及临床病程。
回顾性分析了2家大学医院的数据，并评估了3例自身免疫性脑炎伴N型电压门控钙通道抗体患儿的记录。
3例儿科患者（均为女性）症状涵盖临床谱。然而，3例均有表达性语言退化及易激惹。3例患者的神经影像学检查均正常；3例患者的脑电图（EEG）表现各异。其脑脊液中发现针对N型电压门控钙通道抗体的阳性滴度。静脉注射免疫球蛋白后，3例患者的核心症状均有改善。
儿科人群中与N型电压门控钙通道抗体相关的自身免疫性脑炎临床表现多样，尽管表达性语言延迟和易激惹似乎是常见症状。静脉注射免疫球蛋白治疗可改善核心症状。
